Otosclerosis

Definition

Otosclerosis is an abnormal bone growth in the middle ear that causes hearing loss.

Alternative Names

Otospongiosis

Causes

Otosclerosis is an inherited disorder, which means it is passed down through families. In this condition, an abnormal sponge-like bone grows in the middle ear. This growth prevents the ear from vibrating in response to sound waves -- which must happen in order for you to hear. This lack of vibration leads to hearing loss that continues to get worse with time.

Otosclerosis is the most frequent cause of middle ear hearing loss in young adults. It affects about 10% of the population of the United States, and is most common seen in women ages 15 to 30. Otosclerosis usually affects both ears.

Risks include pregnancy (which may trigger onset) and a family history of hearing loss. Caucasians are more susceptible than others to otosclerosis.

Symptoms

• Hearing loss
  • Slow hearing loss that continues to get worse
  • Hearing may be better in noisy environments than quiet areas.
• Ringing in the ears (tinnitus)

Exams and Tests

A hearing test (audiometry /audiology) may determine the extent of hearing loss. Temporal-bone CT may be used to distinguish otosclerosis from other causes of hearing loss.

Treatment

Otosclerosis may slowly get worse. The condition may not require treatment until you having severe hearing problems.

Medications such as oral fluoride, calcium, or vitamin D may help to control the hearing loss, but the benefits have not yet been proven.

A hearing aid may be used to treat the hearing loss. This will not cure or prevent hearing loss from getting worse, but may help relieve some of the symptoms.

Surgery to remove part of the ear (stapes) and replace it with a prosthesis can cure the condition. A total replacement is called a stapedectomy. Sometimes a laser is used to make a hole in the stapes to allow placement of the prosthetic device. This is called a stapedotomy.

Outlook (Prognosis)

Otosclerosis is progressive without treatment; however, surgery may restore at least partial hearing. Most complications of surgery correct themselves within a few weeks.

To reduce the risk of complications after surgery:

• Do not blow your nose for 1 week after surgery.
• Avoid people with respiratory or other infections.
• Protect the ears against cold.
• Avoid bending, lifting, or straining, which may cause dizziness.
• Avoid loud noises or sudden pressure changes such as scuba diving, flying, or driving in the mountains for 6 months or until healed.

If surgery is unsuccessful, total hearing loss may occur. Treatment then involves developing skills to cope with deafness, including use of hearing aids and visual cues.

Possible Complications

• Complete deafness
• Infection, dizziness, pain, or blood clot in the ear after surgery

When to Contact a Medical Professional

Call your health care provider if hearing loss occurs.

Call your health care provider if fever, ear pain, dizziness, or other symptoms develop after surgery.